title: Aortic disease in patients with Marfan syndrome: aortic volume assessment for surveillance.
abstract: OBJECTIVE
  To assess the reproducibility of aortic volume estimates and to serially test their use in patients with Marfan syndrome.
  
  
  METHODS
  The study was approved by the medical ethics committee and all subjects gave written informed consent. In 81 patients with Marfan syndrome and seven healthy control subjects, aortic volumes and diameters at baseline were estimated by means of contrast material-enhanced magnetic resonance (MR) imaging. At 3 years of follow-up, aortic expansion rate were calculated in a subgroup of 22 patients with Marfan syndrome. Total aortic volume was defined as volume measurement from the level of the aortic annulus to the aortic bifurcation. Intra- and interobserver agreement of aortic volume were calculated by using the intraclass correlation coefficient. Differences in variables were analyzed with the Student t test and logistic regression. Effect size was calculated.
  
  
  RESULTS
  Intra- and interobserver agreement of aortic volume calculation was 0.996 and 0.980, respectively. Mean aortic volume was significantly greater in patients with Marfan syndrome than in control subjects (104 mL/m(2); 95% confidence interval [CI]: 95, 114 mL/m(2) vs 74 mL/m(2); 95% CI: 62, 87 mL/m(2); P < .001). In 22 patients with Marfan syndrome, mean aortic volume was increased at 3 years of follow-up (17 mL; 95% CI: 8, 26 mL; P = .001; effect size, 0.29), while mean aortic diameter did not increase significantly (0.4 mm; 95% CI: 0.0, 0.9 mm; P = .171; effect size, 0.13).
  
  
  CONCLUSIONS
  Assessment of aortic volume is highly reproducible and may be suited for use in the detection of aortic expansion in patients with Marfan syndrome.
  
  
  BACKGROUND
  http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.13122310/-/DC1.
authors.affiliation: Departments of Cardiology, Clinical Epidemiology and Biostatistics, Biomedical Engineering and Physics, and Radiology, Academic Medical Center, Meibergdreef 9, B2-216, 1105 AZ Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands; Department of Cardiology, Radboud University Medical Center, Nijmegen, the Netherlands; Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Alexander W
authors.initials: AW
authors.lastname: den Hartog
authors.forename: Romy
authors.initials: R
authors.lastname: Franken
authors.forename: Piet
authors.initials: P
authors.lastname: de Witte
authors.forename: Teodora
authors.initials: T
authors.lastname: Radonic
authors.forename: Henk A
authors.initials: HA
authors.lastname: Marquering
authors.forename: Wessel E
authors.initials: WE
authors.lastname: van der Steen
authors.forename: Janneke
authors.initials: J
authors.lastname: Timmermans
authors.forename: Arthur J
authors.initials: AJ
authors.lastname: Scholte
authors.forename: Maarten P
authors.initials: MP
authors.lastname: van den Berg
authors.forename: Aeilko H
authors.initials: AH
authors.lastname: Zwinderman
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
authors.forename: Maarten
authors.initials: M
authors.lastname: Groenink
mesh_terms: D000328:Adult; D001018:Aortic Diseases / Q000175:diagnosis* / Q000209:etiology*; D016022:Case-Control Studies; D003287:Contrast Media; D005260:Female; D006801:Humans; D007090:Image Interpretation, Computer-Assisted; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D011159:Population Surveillance; D015203:Reproducibility of Results
chemical_list: D003287:Contrast Media